An outline of the neuropathology of transmissible spongiform encephalopathies (prion diseases)

Paweł P Liberski, James W Ironside

Research output: Contribution to journalArticlepeer-review

Abstract

We review here the basic neuropathology of transmissible spongiform encephalopathies (TSE) or prion diseases. The classic hallmark of TSE neuropathology is a combination (in different proportions in different diseases) of spongiform change, astrocytosis, neuronal loss and amyloid plaques. Immunohistochemically, accumulation of the abnormal isoform of prion protein (PrP(sc) or PrP(d)) is regarded as a diagnostic for TSE. We also review the peculiarities of kuru, variant Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker disease.

Original languageEnglish
Pages (from-to)39-58
Number of pages20
JournalFolia Neuropathologica
Volume42 Suppl B
Publication statusPublished - 2004

Keywords

  • Animals
  • Brain
  • Humans
  • Prion Diseases

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